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Kienbcks Disease
Pedro K. Beredjiklian, MD Osteonecrosis of the lunate, Kienbcks disease, is a progressive, debilitating disease process that can lead to chronic pain and dysfunction. Despite its recognition almost 100 years ago, the etiology remains unidentied, although mechanical, vascular, and traumatic factors have been implicated. The natural history of this disease is poorly dened, and the radiographic appearance does not always correlate with the clinical ndings. Some progress has been made in the identication and an understanding of the progression of the avascular process and its deleterious effects on wrist mechanics. Initial treatment is nonsurgical. Advances in surgical techniques with vascularized pedicled grafts from the distal radius may lead to an improvement in outcomes for patients in the earlier stages of disease, although much more work is needed to determine whether this surgical option represents an improvement over conventional treatment alternatives. Recent reports of long-term outcomes of radial shortening osteotomy for earlier stages of osteonecrosis and proximal row carpectomy for advanced Kienbcks disease reveal that these procedures provide reliable options for the long-term management of this difcult clinical problem. (J Hand Surg 2009;34A:167175. 2009 Published by Elsevier Inc. on behalf of the American Society for Surgery of the Hand.) Key words Kienbcks osteonecrosis, lunate, avascular necrosis. the general population.2 This study led to a longstanding belief in an association between ulnar negative variance and avascular necrosis of the lunate, and it bolstered proponents of a mechanical etiology of the disease. More recent studies evaluating patients of Asian ethnicity have shown that Kienbcks disease can occur in patients with ulnar neutral and ulnar positive variance.3 ETIOLOGY The lunate is the central bone of the proximal carpal row and articulates proximally with the lunate fossa of the radius and triangular brocartilage complex and distally with the capitate and hamate. The lunate and the lunate fossa of the radius are both hemispherical in shape and have a high degree of congruence between the articular surfaces. This anatomic factor is believed to play an important role in the nding that the radiolunate articulation is one of the last joints in the wrist to develop degenerative changes. Because Kienbcks disease is fundamentally an avascular process, the patterns of blood supply to the lunate have received much attention and may shed some light into the development of osteonecrosis.4,5 Multiple patterns of blood supply have been identied

BRIEF HISTORICAL BACKGROUND Robert Kienbck, an Austrian radiologist, provided the rst description of avascular necrosis of the lunate.1 His treatise Concerning Traumatic Malacia of the Lunate and Its Consequences was published in 1910. In this work, Kienbck described an isolated disease of the lunate associated with secondary changes in the other carpal bones. Patients presented with pain, loss of mobility, and prominence in the area of the wrist. Radiographic evaluation revealed isolated changes in the proximal aspect of the lunate, with eventual collapse and fragmentation. This process was believed by Kienbck to occur due to a disturbance in nutrition of the lunate secondary to repetitive trauma. In 1928, Hultn, in an evaluation of 23 Swedish patients with Kienbcks disease, noted that 78% had an ulnar negative variance, compared with only 23% of
From the Rothman Institute, Philadelphia, PA. Received for publication October 12, 2008; accepted October 12, 2008. No benets in any form have been received or will be received related directly or indirectly to the subject of this article. Corresponding author: Pedro K. Beredjiklian, MD, Rothman Institute, 925 Chestnut Street, 5th Floor, Philadelphia, PA 19107; e-mail: info@rothmaninstitute.com. 0363-5023/09/34A01-0028$36.00/0 doi:10.1016/j.jhsa.2008.10.012

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in cadaveric specimens, but most receive nutritional supply from dorsal and volar arterial vessels.5 These blood vessels enter the bone through the nonarticular surfaces. In 1 study, 7% to 23% of lunates received their blood supply from a single volar arterial source.4,5 When the vessel or vessels enter the bone, several intraosseous branching congurations have been observed, including I, X, and Y patterns. It has been postulated that lunates with a single arterial nutrient or those with limited intraosseous branching patterns may be more susceptible to the development of osteonecrosis from acute or chronic repetitive trauma. In addition, the uniform presence of a volar vascular supply explains why patients with perilunar instability and dislocation seldom develop avascular changes in the lunate, as the volar capsular attachments to the lunate remain intact.6 Disruption of venous outow leading to increased intraosseous pressure could also lead to avascular changes and osteonecrosis. In vitro intraosseous pressures have been found to be increased in necrotic lunates compared with normal lunates. One study recently examined the venous drainage from cadaveric specimens.7 The authors found a dense plexus of small venous vessels at the volar and dorsal periosteal surface of the bones. The authors assert that the plexus could be a site of venous outow disruption, secondary to systemic and local compressive factors. Mechanical factors have also been theorized to contribute to the development of osteonecrosis.8,9 As mentioned earlier, the height relationship between the radius and the ulna at the distal radioulnar and radiocarpal joints (ulnar variance) has long been thought to play a role in this condition. Other mechanical factors, such as radial inclination, are thought to contribute to the process, particularly in patients with ulnar neutral or ulnar positive variance in which increased radiolunate stresses secondary to ulnar negative variance would not account for the development of the avascular process. It has been noted that patients with decreased radial inclination have a tendency toward smaller lunates and an association with Kienbcks disease.8 Systemic factors have also been associated with lunate osteonecrosis. Conditions in which there is a tendency to hypercoagulability, decreased arterial inow, or increased venous congestion have been thought to play a role in this process.6 Systemic corticosteroid use, sickle cell disease, cerebral palsy, and septic emboli have all been associated with osteonecrosis, although there is no dened correlation between these systemic conditions and lunate osteonecrosis. At this point, nearing 100 years after Kienbcks seminal work, the eti-

ology remains undened. Although mechanical, anatomic, and systemic mechanisms have all been implicated in the development of the disease process, no specic etiologic mechanism has been identied. It is most likely that a complex interrelationship between all of the factors described earlier contribute to the disease process.6,8,9 CLINICAL AND RADIOGRAPHIC FINDINGS Patients affected with Kienbcks disease typically present with pain and weakness in the wrist, often without a history of acute trauma. Symptoms may be present a variable amount of time before presentation, although patients will often complain of longstanding symptoms that are progressive. For this reason, the natural history of this condition is not well known. The pain can be described from just mild and occasional to severe and debilitating. It is most common in young adult men (age 20 40) and is rare in children, although some case reports have documented pediatric involvement. It is rarely bilateral. On examination, there is localized dorsal wrist swelling about the lunate, likely due to synovitis, which is painful on palpation. There is almost always decreased range of motion of the wrist joint, with loss of exion and extension. There is typically pain at the end range of motion, particularly with extension. Forearm rotation is typically preserved. There is also considerable loss of grip strength compared to the contralateral, unaffected side. Radiographic studies are diagnostic for Kienbcks disease. Depending on the disease stage, radiographs are evaluated for diffuse sclerosis of the lunate, cystic changes, fragmentation, articular surface collapse, and perilunate arthritic changes. Often, a fracture can be visualized on the lateral x-ray in the early stages of disease, and it is unclear whether this represents an actual injury or whether the fracture line is the result of the decreased structural properties of the osteonecrotic bone. X-rays are also of importance in determining associated anatomic and mechanical properties of the involved wrist, such as ulnar variance, radial inclination, carpal height, radioscaphoid angle, and the size of the lunate. Magnetic resonance imaging (MRI) is an important adjunct to diagnosis. It is particularly helpful in the early stages of the disease, when ndings may be absent on plain lms. On the T1-weighted images, loss of marrow fat causes decreased signal intensity. Similarly, T2-weighted images also reveal low signal intensity.6 In order to establish the diagnosis of Kienbcks disease, it is imperative that the signal changes are diffuse

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TABLE 1. Disease
Stage 1 Stage 2 Stage 3 Stage 3A Stage 3B Stage 4

Lichtman Classication of Kienbcks

Normal x-ray, signal intensity changes on MRI Lunate sclerosis on plain x-ray; fracture lines may be present Collapse of the lunate articular surface Normal carpal alignment and height Fixed scaphoid rotation, proximal capitate migration, loss of carpal height Lunate collapse along with radiocarpal or midcarpal arthritis

Modied from Allan CH, Joshi A, Lichtman DM. Kienbocks disease: diagnosis and treatment. J Am Acad Orthop Surg 2001;9:128 136.

STAGING (TABLE 1) The staging of Kienbcks disease depends primarily on the radiographic ndings. Staging is a critical part of the evaluation and management of patients affected with this condition, as it dictates the various treatment options applicable in each case. Several classication schemes have been suggested, but the one described by

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and involve the entirety of the lunate. In cases in which the signal intensity changes are localized to a specic area of the bone, other diagnoses should be considered. For example, if the changes are conned to the proximal-ulnar aspect of the lunate, the diagnosis of ulnocarpal abutment must be strongly considered. Other diagnostic studies can also be of help. Before the advent of MRI, technetium bone scanning was useful for diagnosis in cases with negative x-rays. Finally, spiral or computed tomography can be helpful in the assessment of articular surface collapse and the presence of fractures. In cases in which the plain x-rays do not reveal obvious signs of radiocarpal or midcarpal articular joint involvement, diagnostic arthroscopy can also be a helpful adjunct in the staging of the disease process. Bain and Begg have described an arthroscopic classication scheme based on the number of articular surfaces involved.10 The classication system classies the perilunar articular surfaces (proximal lunate, distal lunate, lunate facet of the radius) as functional or nonfunctional, and a treatment algorithm is provided according to the arthroscopic ndings. Arthroscopy in this setting should be used sparingly, as plain x-rays and the other radiographic studies are almost always diagnostic in terms of the assessment of the joint surfaces.

Lichtman et al. is the most commonly used and one that has shown to have good reproducibility and reliability.6 In stage 1, plain x-rays reveal normal density and articular surfaces of the lunate, but MRI will reveal diffuse signal intensity changes (Fig. 1). In stage 2, the lunate appears diffusely sclerotic on plain x-ray, and fracture lines may be present, although there is preservation of the articular surfaces (Fig. 2). Stage 3 is the most common stage on initial presentation and is dened by collapse of the lunate articular surface. This stage is subdivided, with stage 3A revealing articular collapse but with the maintenance of normal carpal alignment and height. Stage 3B, in contrast, is dened by the presence of articular collapse and xed scaphoid rotation, proximal capitate migration, and decreased carpal height (Fig. 3) Finally, stage 4 is dened by lunate collapse associated with radiocarpal or midcarpal arthritis. In a study evaluating the reliability of the Lichtman classication, Goldfarb and colleagues found that this classication overall had substantial interobserver reliability.11 However, the authors also found that the differences between stages 3A and 3B were less reliably identied. For this reason, they proposed a new modication of the classication system in which stage 3B was dened as one in which there was a radioscaphoid angle of more than 60. With the modication of the staging system, the interobserver reliability increased both for the overall classication (from kappa 0.63 to 0.81) and the stage 3A (from kappa 0.38 to 0.75). As a result, the authors concluded that the radioscaphoid angle increases the interobserver reliability of the Lichtman classication and the ability to more reliably distinguish between stages 3A and 3B disease. TREATMENT (TABLE 2) The treatment for Kienbcks disease is primarily guided by the patients symptomatology and functional decits, as well as by the disease stage as outlined earlier. Most longitudinal studies evaluating the conservative management of Kienbcks disease have identied radiographic progression of disease through the stages as described earlier.1214 Recently, Keith and colleagues evaluated 33 patients treated nonsurgically for Kienbcks disease.14 They identied loss of range of motion (exion) and a decrease in functional status as shown by a decline in scores in the Disabilities of the Arm, Shoulder, and Hand questionnaire. Even though the longitudinal radiographic assessment was limited for their patient series, the authors concluded that osteonecrosis of the lunate is a progressive disease. It is controversial whether surgical intervention

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FIGURE 1: A Posteroanterior x-ray and B T1-weighted coronal MRI of a patient with stage 1 Kienbcks disease.

FIGURE 2: Posteroanterior x-ray of a patient with stage 2 disease. Note sclerosis, fracture of the body of the lunate but intact articular surfaces.

FIGURE 3: Posteroanterior x-ray of a patient with stage 3B disease. Note the articular collapse and xed rotatory deformity of the scaphoid.

yields better outcomes than conservative management. Delaere and colleagues compared night splinting to surgery in patients with up to stage 3 disease.15 In this study, immobilization yielded equivalent results to surgery. These results should be considered carefully in light of the fact that, on average, the immobilization group had a lower stage of disease than the surgical group. Other studies indicate that surgical intervention is superior to nonsurgical management. Salmon et al. provide evidence that radial shortening osteotomy may be superior to nonsurgical management.12

Despite the disease progression, many patients, even with advanced Kienbcks disease, remain functional and with tolerable symptoms. Therefore, a trial of conservative treatment with splinting or casting and adjunctive analgesic or anti-inammatory medication appears to be warranted in most patients, regardless of the stage at presentation. Splinting should be as long as 3 months.6 Surgical intervention should be reserved for patients who fail a trial of conservative management and remain symptomatic.

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TABLE 2. Treatment of Kienbcks Disease According to Stage

Stage 1 Stage 2 to 3A, ulnar negative variance Stage 2 to 3A, ulnar positive variance Stage 3B Cast immobilization for 3 months Radial shortening; ulnar lengthening; capitate shortening Vascularized bone graft and external xation; radial wedge or dome osteotomy; capitate shortening Intercarpal arthrodesis (scaphotrapezio-trapezoidal, scaphocapitate); lunate excision; radial shortening; proximal row carpectomy Proximal row carpectomy; wrist arthrodesis; wrist denervation

Stage 4

There are multiple surgical procedures for the treatment of osteonecrosis of the lunate, and the literature casts little evidence-based information to determine which surgical choices are better than others. Despite the multiple surgical choices available, there is not one specic approach that has been shown superior. Using the previously described staging scheme as a guide, different procedures are indicated for different stages. Anatomic variables such as ulnar variance and radial inclination play a large role in the determination of which procedure is indicated for each individual patient. Stage 1 As in all cases, immobilization in a splint or cast is the rst option for treatment. External xation has been used both as an immobilization device and also to unload the lunate, although the experience with this approach is limited.16 Although some patients improve with conservative management, it is generally believed that many of these patients progress to stage 2 despite treatment.6 Stages 2 and 3A These stages are generally considered together with regard to treatment options, where the main goal is to revascularize the lunate in order to prevent progression. The one exception to this grouping is direct revascularization procedures, used mostly for patients in stage 2 when articular collapse has not occurred. Nevertheless, many authors have used these procedures where there is already some articular disruption, in what has been termed early stage 3A Kienbcks. Several types of revascularization procedures have been described for the treatment of stage 2 disease.1722

These include vascularized transfers of the pisiform bone, vascularized pedicled transfers from the distal radius (including pronator quadratus), direct implantation of metacarpal arteries, and free vascularized grafts, among others. In addition to the vascularized transfer, an unloading procedure to decrease mechanical stresses on the lunate (external xation, pinning of the scaphotrapezio-trapezoidal [STT] or scaphocapitate joints) is commonly performed concomitantly.20,21 Vascularized pedicled bone transfers from the dorsal distal radius have recently gained interest.19 Several types of grafts have been described, based on their location with respect to the extensor compartments of the wrist and also as a function of whether the feeder vessel is located outside or within the extensor compartment. The graft most commonly described for the treatment of Kienbcks disease is based on the 4 5 extracompartmental artery. Retrograde blood ow from the 5th extracompartmental artery is directed in an anterograde direction into the 4th extracompartmental artery by ligation of the posterior branch of the anterior intraosseous artery. This graft provides a large pedicle diameter and long pedicle length, and it is located ulnarly. Moran et al. recently evaluated their experience with 26 patients followed up for an average of 31 months.22 The vast majority of patients experienced pain relief and improved grip strength. Of this patient group, 71% of patients exhibited evidence of lunate revascularization. Despite the overall success of the procedure, 23% of patients showed radiographic evidence of disease progression at the latest follow-up. Vascularized pisiform bone transfers are performed by harvesting the ipsilateral pisiform bone, along with its blood supply, from small dorsal and radial branches of the ulnar artery. The pedicled bone graft is then inserted into a defect created surgically on the dorsal surface of the lunate. Daecke and colleagues recently described their experience with 23 patients treated for stage 2 or 3A with this procedure and followed up for an average of 12 years.18 Pain and function improved in 20 of 23 patients. Of the 20 patients in which preoperative x-rays were available, progression of the disease was seen in 6 (33%). Free vascularized transfers have also been used to treat osteonecrosis of the lunate. Gabl and colleagues evaluated 18 patients with stage 3 disease treated by implantation of a free vascularized corticocancellous iliac bone graft.23 The wrist was stabilized with an external xator during healing. At a mean follow-up of 5 years, the graft became incorporated into the lunate in 16 patients, and no carpal collapse occurred

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in these cases. The graft did not integrate and was resorbed in the other 2 patients. In a follow-up study, Arora et al. found that at the 10-year follow-up, the 16 patients still had no evidence of disease progression or carpal collapse.24 Other options for patients in these stages of disease include the joint-leveling procedures.6,8,9 These procedures are believed to mechanically unload the lunate, leading to revascularization and prevention of progression of the articular surface collapse. The determination of which procedure is adequate for each patient depends primarily on the length relationship between the radius and the ulna at the wrist. In patients with ulnar negative variance, leveling the distal radioulnar joint to neutral or about 1 mm of ulnar positive variance is the goal. This goal can be achieved by either shortening the radius or lengthening the ulna. Ulnar lengthening requires bone grafting, and there is an increased chance of nonunion; therefore, it is not commonly performed.8,9 Radial shortening osteotomies are technically simple and have a low incidence of nonunion (Fig. 4). Several authors have recently described long-term follow-up studies detailing the outcomes of this procedure. Raven et al. followed 12 patients treated in this fashion for an average of 22 years.25 One patient had stage 3B disease, and the rest had stage 2 or 3A disease. Only 3 patients showed mild progression of disease at the latest follow-up, and most had excellent pain relief and function. In a similar study, Watanabe et al. evaluated 12 patients treated with a radial shortening osteotomy at a mean follow-up of 21 years.26 Most patients had mild wrist pain and maintained good range of motion and function, but x-rays revealed disease progression in 50% of the patient group. Radial shortening osteotomies in patients with stage 3B disease remain controversial. Altay and colleagues recently compared the effectiveness of the procedure in patients with stages 3A (n 13) and 3B (n 10) at an average follow-up period of 85 months.27 The authors found no notable differences in pain and range of motion between stages, and they concluded that this procedure has clinically similar outcomes regardless of stage, despite the lack of radiological improvement. Joint leveling options for patients with ulnar neutral or ulnar positive variance include radial closing-wedge osteotomy, radial dome osteotomy, and capitate shortening osteotomy with or without capitohamate arthrodesis (Fig. 5).28 The radial osteotomies are used to reduce the radial inclination of the articular segment in an effort to reduce the radiolunate contact stresses. This

is achieved by increasing the contact area between the radius and lunate and decreasing the force across the radiolunate and capitolunate joints.28 Koh et al. reported improvements in pain, motion, and function at a minimum 10-year follow-up.29 However, arthritic changes were seen in almost three quarters of the patients at the latest follow-up, suggesting that disease progression was not halted. The goal of these procedures is to unload the articular surfaces of the lunate by shifting the load to adjacent carpal articulations. These procedures generally provide similar outcomes as the radial shortening osteotomy, with good pain relief, restoration of motion and function, and possible halting or slowing disease progression. Core decompression of the radius and ulna is a novel concept that has been described for patients with stages 1 to 3A. Illaramendi et al. reported their experience with this procedure in 22 patients at an average follow-up of 10 years.30 This procedure is performed by decompressing the distal metaphyses of the radius and ulna through small cortical windows, using a curette. The authors found good restoration of motion and pain, with only 4 patients with complaints of mild pain. Most of the patients (17 of 22) did not show disease progression at latest follow-up, 2 improved radiographically, and 3 progressed. Stage 3B In this stage, there is loss of carpal height due to exion of the scaphoid, as well as articular collapse of the lunate articular surface. Correction of the exed scaphoid along with intercarpal arthrodesis (STT or scaphocapitate) can decrease load on the lunate, helps to stabilize the midcarpal joint, and can prevent further carpal collapse. In addition to the arthrodesis, the lunate can be removed and replaced with a tendon anchovy or a lunate implant to diminish joint irritation. Van den Dungen et al. recently compared the outcomes of patients treated conservatively (n 19) and with STT arthrodesis (n 11) with a mean follow-up of 13 years. The authors state that the 2 groups were statistically comparable, and found that STT arthrodesis led to an increased loss of mobility and pain and a longer rehabilitation time as compared to conservative treatment.31 Tambe and colleagues compared outcomes of patients treated with partial intercarpal arthrodesis and radiocarpal arthrodesis for advanced Kienbcks disease, and they found that patients treated with radiocarpal arthrodesis had improved outcomes at latest follow-up.32 Proximal row carpectomy (PRC) is a successful salvage procedure, but the capitate head must be largely

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FIGURE 4: A Posteroanterior x-ray, B lateral x-ray, C T1-weighted, and D T2-weighted coronal MRI of a patient with stage 3A Kienbcks disease. Note articular collapse but the absence of carpal collapse or rotation of the scaphoid. The patient displays ulnar negative variance. E Postoperative posteroanterior x-ray of the patient following radial shortening osteotomy. JHS Vol A, January

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FIGURE 5: Posteroanterior x-ray of a patient with stage 3A disease treated with a capitate shortening osteotomy.

free of degenerative changes. If mild arthritic changes are present on the capitate head, an interpositional arthroplasty of dorsal wrist capsule in addition to the PRC can be effective.33 Several studies have detailed long-term outcomes of PRC for the treatment of Kienbcks disease. Croog and Stern described a series of 21 patients treated for stages 3 and 4 disease at an average 10-year follow-up.34 Three patients in the series required radiocapitate arthrodesis due to persistent pain, and 2 of the 3 patients had stage 4 disease at the time of the index procedure. The authors concluded that PRC is a reliable procedure in this setting, but caution about performing the procedure in patients with advanced disease. In a similar study, DiDonna et al. detailed their experience with PRC at a minimum 10-year follow-up for patients with degenerative conditions of the wrist, including Kienbcks disease.35 Again, the authors concluded that PRC is a reliable procedure in this setting, although caution is given to performing this procedure in patients younger than 35 years of age. De Smet and colleagues reported on 21 patients with advanced Kienbcks disease at a mean follow-up of 67 months.36 Finally, Lumsden et al. report on 13 patients at an average 15-year follow-up.37 Like the previous authors, these authors found this procedure to be a reliable longterm approach to the treatment of advanced Kienbcks disease.

Stage 4 The mainstays of treatment for this stage include PRC and wrist arthrodesis. Proximal row carpectomy and wrist arthrodesis are the mainstays of treatment. As mentioned earlier, mild arthritic changes on the capitate head can be addressed at the time of PRC with an interpositional arthroplasty of dorsal wrist capsule. Nevertheless, arthritic changes in this region are likely to lead to continued pain and failure of the procedure, necessitating further intervention in the form of arthrodesis.34 Wrist denervation alone or in combination with the aforementioned procedures can help with symptom relief. Schweizer and colleagues reported their long-term experience with complete wrist denervation in a series of 70 patients, 11 of whom had Kienbcks disease.38 The average follow-up in this series was 9.6 years. Complete wrist denervation led to subjective improvement in about two thirds of patients, and about one half reported complete or marked resolution of the pain. Kienbcks disease remains a challenging clinical problem for practicing hand surgeons. The etiologic factors behind the disease process remain unidentied. Recent surgical advances in the form of local vascularized pedicled grafts from the distal radius have the potential to improve the treatment of this condition. Traditional procedures for treatment of advanced disease, such as radial shortening osteotomy and PRC, have shown in long-term studies to provide adequate symptom control and functional restoration. REFERENCES
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